Hematology

获得性血栓性血小板减少性紫癜

Acquired Thrombotic Thrombocytopenic Purpura

Acquired Thrombotic Thrombocytopenic Purpura (aTTP), also known as Moschcowitz syndrome, is a subtype of thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multiorgan ischemic damage caused by ADAMTS13 deficiency. / 获得性血栓性血小板减少性紫癜（aTTP），又称Moschcowitz综合征，是一种血栓性微血管病亚型，其特征为微血管病性溶血性贫血、血小板减少和多器官缺血性损伤，由ADAMTS13缺乏引起。

Knowledge type: 自然理论
First proposed or discovered
Proposer: Turkey Farm member
Creator: 农场主
Last updated: 2026-10-28 21:01:25

Sub-knowledge points

Microangiopathic hemolytic anemiaThrombocytopeniaMultiorgan ischemic damageADAMTS13 deficiencyAutoimmune mechanismIgG family antibodies against ADAMTS13

Higher-order knowledge points

Thrombotic microangiopathyvon Willebrand factor (vWF) multimersKidney transplantation complications

Main parameters and performance indicators

ADAMTS13 activity: 0.01 U/mL (severe deficiency)ADAMTS13 inhibitor titer: 19.34 BU (positive)PLASMIC score: 5 (intermediate risk)Hemoglobin: 7.5 g/dL (anemia)Platelets: 18,000/mmc (thrombocytopenia)Schistocytes: 1.6% (microangiopathic hemolysis)LDH: 709 U/L (elevated)Indirect bilirubin: 2.3 mg/dL (elevated)Haptoglobin: 0.3 g/L (undetectable)

Open problems

Diagnostic masking by post-operative complicationsDrug-induced microangiopathic hemolytic anemia (TMA) differentiationTriggering mechanism of pre-existing silent autoimmune state

Related literature

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获得性血栓性血小板减少性紫癜

Sub-knowledge points

Higher-order knowledge points

Main parameters and performance indicators

Open problems

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